In this context, we have recently turned to canine degenerative myelopathy (DM), a multisystem central and peripheral axonopathy described in dogs in 1973 (Averill, 1973), with an overall prevalence of 0.19% (Coates and Wininger, 2010 for review), which shares pathogenic mechanisms with some forms of human ALS, including mutations in SOD1 as one of the major causes of the disease (Awano et al., 2009). Here, SOD1 is linked to amyotrophic lateral sclerosis.